Severe Ischemic Manifestations Research Articles

A Retrospective Study of Chinese Patients With Giant Cell Arteritis (GCA): Clinical Features and Factors Associated With Severe Ischemic Manifestations.

A retrospective study was performed on 70 giant cell arteritis (GCA) patients in Peking Union Medical College Hospital (PUMCH). The aim of this study was to describe the clinical features of these Chinese GCA patients and explore the possible associated factors for severe ischemic manifestations. Medical charts of all patients were reviewed, and the demographic, clinical, and laboratory data were analyzed. The mean age at disease onset was 65.2 years old, and the ratio of male to female was 1:1. Fever and headache were most prominent symptoms at onset, which occurred in 51.4% and 30.0% of patients, respectively. Common manifestations at diagnosis were constitutional symptoms (85.7%), headache (68.8%), visual impairment (38.6%), jaw claudication (30%), scalp tenderness (30%), and concurrent polymyalgia rheumatic (27.1%). No significant difference in clinical manifestations between genders was observed. Comparisons between patients with and without severe ischemic manifestations including jaw claudication, permanent visual loss, or cerebrovascular accident had shown that fever and asthenia were significantly less frequent in patients with severe ischemic manifestations (P = 0.006 and 0.023, respectively), and the mean value of erythrocyte sedimentation rate (ESR) was significantly lower in patients with severe ischemic manifestations than patients without (P = 0.001). History of smoking was more frequent in patients with severe ischemic manifestations (P = 0.038).This is the largest group of GCA patients from China so far. When compared our data with patients reported in the literature, this series of GCA patients were younger and without female predominance. The clinical manifestations of patients in this report were similar to other studies except for a higher prevalence of constitutional symptoms. The results of this study indicated that lower systemic inflammatory response and the history of smoking might be associated with severe ischemic damages.

Total occlusion of the abdominal aorta in a patient with renal failure and refractory hypertension: a case report

Total occlusion of the abdominal aorta is unusual, and potentially catastrophic. It occurs in patients with advanced atherosclerotic occlusive disease, and can cause severe ischemic manifestations, depending on the site of obstruction. Prompt and appropriate diagnostic and therapeutic approaches are important whenever this condition is suspected, in order to avoid a fatal outcome. The development of a complex network of collaterals may prevent the manifestation of acute ischemic phenomena, and cause a delay in diagnosis and treatment. Here we report the clinical case of a 59-year-old man who was referred to our Department for evaluation of renal failure and refractory hypertension. Ultrasonography and 99mTc-DTPA scintigraphy showed a shrunken, non-functioning left kidney, while CT angiography and aortography showed the complete occlusion of the aorta from below the right renal artery down to the bifurcation of both common iliac arteries, with a critical stenosis of the origin of the right renal artery, an occlusion of the left renal artery as well as of the origin of the inferior mesenteric artery. The patient was referred to the surgery department for aorto-bifemoral bypass surgery and re-implantation of the right renal artery.

Acute ascending thrombosis of abdominal and suprarenal aorta.

We report the diagnostic and successful therapeutic images of an acute occlusion of the abdominal and suprarenal aorta. This lesion is a rare but catastrophic pathology which can cause severe ischemic manifestations, depending on the site of obstruction, with high rate of mortality even after treatment. In the majority of cases it represents a surgical emergency. Although the mechanism of the thrombosis has not been delineated, the proposed etiologies include propagation of thrombus from distal artery occlusion, cardiac thromboembolism, dislodgment of a mural thrombus, or coagulation disorders. Frequent risk factors include advanced atherosclerosis combined with a low flow state because of poor cardiac performance. The management of this condition includes immediate intervention with systemic heparinization, improvement of the cardiac condition, and surgical revascularization based on the clinical and anatomical presentation. In this case the authors highlight the importance of an early detection and early intervention to enhance survival rates and reduce morbidity.

Lack of Association Between the rs6920220 (G/A) Polymorphism of the 6q23 Region and Biopsy-proven Giant Cell Arteritis

Recently, 2 independent studies have identified an association between several single-nucleotide polymorphisms (SNP) located in the 6q23 chromosomal region and rheumatoid arthritis (RA). Like RA, giant cell arteritis (GCA) is also a complex polygenic disease in which more than 1 genetic locus is likely to contribute to disease susceptibility and clinical expression. We analyzed the involvement of the rs6920220 (G/A) polymorphism from the 6q23/TNFAIP3 gene region in susceptibility to GCA. Two hundred twenty patients with biopsy-proven GCA and 490 matched controls were assessed. DNA from patients and controls was obtained from peripheral blood. Samples were geno-typed for the 6q23 region rs6920220 using a TaqMan allele discrimination assay and by polymerase chain reaction (PCR) amplification. After PCR, the genotype of each sample was attributed automatically by allelic-specific fluorescence using the ABI Prism 7900 sequence detection system. No significant differences in the genotype distribution between patients with GCA and controls for the rs6920220 (G/A) polymorphism were found. No significant differences were observed when patients with GCA were stratified according to the presence of specific clinical features of the disease such as polymyalgia rheumatica or severe ischemic manifestations or specific visual ischemic complications. Our results show no involvement of this 6q23/TNFAIP3 gene region SNP in the susceptibility to or clinical expression of GCA.

Role of the C8orf13-BLK region in biopsy-proven giant cell arteritis

Giant cell arteritis (GCA) is a complex polygenic disease in which more than one genetic locus is likely to contribute to disease susceptibility and clinical expression. In the present study, we have analyzed for first time the implication of rs13277113 and rs2736340 variants from the C8orf13-BLK gene region in the susceptibility to GCA. A total of 220 biopsy-proven GCA patients and 486 matched controls were assessed. DNA from patients and controls was obtained from peripheral blood. Samples were genotyped for the C8orf13-BLK region rs13277113 and rs2736340 using a predesigned TaqMan allele discrimination assay. No significant differences in the genotype distribution between GCA patients and controls for the rs13277113 and rs2736340 C8orf13-BLK gene variants were found. GCA patients were also stratified according to the presence of specific clinical features of the disease. In this regard, the allele A of the rs13277113 variant was overrepresented in patients with severe ischemic manifestations compared with patients without severe ischemic manifestations ( p = 0.04; OR =1.65; 95% CI = 0.99–2.78). In conclusion, our results do not support a major implication of the C8orf13-BLK gene region in susceptibility to GCA. However, a potential implication of the rs13277113 variant in the development of severe ischemic complications may exist.

Lack of Association Between STAT4 Gene Polymorphism and Biopsy-proven Giant Cell Arteritis

To investigate the potential implication of the STAT4 gene polymorphism rs7574865 in the predisposition to or the clinical expression of giant cell arteritis (GCA). A total of 212 patients diagnosed with biopsy-proven GCA were studied. DNA from patients and controls matched by age, sex, and ethnicity was obtained from peripheral blood. Samples were genotyped for STAT4 rs7574865 polymorphism. No statistically significant differences in the allele frequencies for the STAT4 rs7574865 polymorphism were observed between patients and controls. Although we observed an increased frequency of the T/T genotype in GCA patients (6.0%) compared to healthy controls (3.9%), this difference did not achieve statistical significance (OR 1.57, 95% CI 0.72-3.41). No statistically significant differences in allele or genotype frequencies were observed when patients were stratified according to the presence of typical disease features such as polymyalgia rheumatica, severe ischemic manifestations, and visual ischemic complications in the setting of this vasculitis. Our results do not support a major role of the STAT4 rs7574865 gene polymorphism in susceptibility to or clinical manifestations of GCA.

Epidemiological and clinical features of giant cell arteritis in Tunisia

Background Giant cell arteritis (GCA) is a systemic vasculitis of the elderly that could result in vision loss or even be life threatening. Unlike western countries, this disease is considered exceptional in Tunisia. Objective The aims of this study were to determine epidemiological and clinical features of GCA in Tunisian population and to identify management difficulties. Patients and methods A multicentric study of 96 patients in whom GCA was diagnosed between 1986 and 2003. All patients fulfilled the ACR criteria for classification of GCA. Results The majority of cases (77%) were diagnosed since 1994. The male/female ratio was 0.88 and the mean age at the time of diagnosis was 70.8 ± 7.7 years. Clinical features were characterized by gradual onset in 64.4% of cases. The most frequent clinical manifestations were headache (91.7%), abnormalities in temporal arteries (85.4%), severe ischemic manifestations (80.2%), constitutional symptoms (75%), and polymyalgia rheumatica (56.3%). Biological inflammatory syndrome was noted in all patients. Temporal artery biopsy established histological diagnosis in 73% of cases. All patients were treated by corticosteroids. Remission was obtained in 45.6%. Relapses occurred in 40.4% of cases and 30 patients were still receiving corticosteroids at the time of study. Four patients died and irreversible ischemic complications were noted in 15.6% of cases. Steroid adverse effects occurred in 56 patients. Conclusion GCA is not exceptional to Tunisia. It occurs amongst elderly patients with no female predominance noticed. Clinical features are similar to those reported in other series.

Giant cell arteritis: laboratory tests at the time of diagnosis in a series of 240 patients

The outcome of a patient with giant cell arteritis (GCA) is closely related to the development of severe ischemic manifestations. In the current study we analyzed the implications of routine laboratory tests obtained at the time of diagnosis in the clinical spectrum of a series of 240 consecutive patients with biopsy-proven GCA at the single hospital for a defined population. We also examined whether the laboratory markers of inflammation may be predictors of severe ischemic manifestations (visual ischemic events, cerebrovascular accidents, jaw claudication, or large-artery stenosis of the extremities of recent onset), and their potential correlation. Anemia (hemoglobin <12 g/dL) was observed in 131 (54.6%) and thrombocytosis in 117 (48.8%) patients. Sixty-eight (28.3%) patients had leukocytosis. The percentage of patients showing a significant increase of alkaline phosphatase and hypoalbuminemia was similar (25% and 27.8%, respectively). The mean values of erythrocyte sedimentation rate (ESR) and C-reactive protein were 93 +/- 23 mm/h and 94 +/- 63 mg/L, respectively. A strong correlation among most laboratory markers of inflammation was observed. Anemia was more commonly observed in patients without severe ischemic manifestations (61.5% versus 48.9% in those with severe ischemic manifestation; p = 0.05) and in patients with constitutional syndrome or fever (p < 0.001). Patients with ESR greater than 100 mm/h exhibited more commonly constitutional syndrome (p < 0.001) and had a statistically significant reduction in the incidence of visual ischemic events (p < 0.025). Only 7 (22.6%) of the 31 patients who suffered permanent visual loss had an ESR at the time of disease diagnosis greater than 100 mm/h. However, in a multivariate logistic regression analysis, only anemia was found to be a negative predictor for the development of severe ischemic manifestations of GCA (odds ratio, 0.53; 95% confidence intervals, 0.30-0.94; p = 0.03). In conclusion, our results suggest that some laboratory markers of inflammation, in particular the presence of anemia, may negatively predict the risk of severe ischemic complications in GCA patients.

Giant Cell Arteritis

The outcome of a patient with giant cell arteritis (GCA) is closely related to the development of severe ischemic manifestations. In the current study we analyzed the implications of routine laboratory tests obtained at the time of diagnosis in the clinical spectrum of a series of 240 consecutive patients with biopsy-proven GCA at the single hospital for a defined population. We also examined whether the laboratory markers of inflammation may be predictors of severe ischemic manifestations (visual ischemic events, cerebrovascular accidents, jaw claudication, or large-artery stenosis of the extremities of recent onset), and their potential correlation. Anemia (hemoglobin <12 g/dL) was observed in 131 (54.6%) and thrombocytosis in 117 (48.8%) patients. Sixty-eight (28.3%) patients had leukocytosis. The percentage of patients showing a significant increase of alkaline phosphatase and hypoalbuminemia was similar (25% and 27.8%, respectively). The mean values of erythrocyte sedimentation rate (ESR) and C-reactive protein were 93 +/- 23 mm/h and 94 +/- 63 mg/L, respectively. A strong correlation among most laboratory markers of inflammation was observed. Anemia was more commonly observed in patients without severe ischemic manifestations (61.5% versus 48.9% in those with severe ischemic manifestation; p = 0.05) and in patients with constitutional syndrome or fever (p < 0.001). Patients with ESR greater than 100 mm/h exhibited more commonly constitutional syndrome (p < 0.001) and had a statistically significant reduction in the incidence of visual ischemic events (p < 0.025). Only 7 (22.6%) of the 31 patients who suffered permanent visual loss had an ESR at the time of disease diagnosis greater than 100 mm/h. However, in a multivariate logistic regression analysis, only anemia was found to be a negative predictor for the development of severe ischemic manifestations of GCA (odds ratio, 0.53; 95% confidence intervals, 0.30-0.94; p = 0.03). In conclusion, our results suggest that some laboratory markers of inflammation, in particular the presence of anemia, may negatively predict the risk of severe ischemic complications in GCA patients.

Giant Cell Arteritis

Classically, patients with giant cell arteritis (GCA) present with cranial ischemic manifestations that are directly related to vascular involvement. However, a variable proportion of GCA patients may present without obvious vascular manifestations. Since a high index of suspicion of this condition in individuals over the age of 50 years is needed to prevent the development of severe complications, we have studied the different patterns of disease presentation in a series of 240 patients with biopsy-proven GCA diagnosed at the single hospital for the well-defined population of Lugo, Spain, between January 1, 1981, and June 15, 2004. During the study period, 203 (86.4%) GCA patients presented with headache. Patients with headache were found to have an abnormal temporal artery on physical examination more commonly than the other GCA patients (79.8% versus 35.1%; p < 0.001). Compared with the other GCA patients, those who presented with polymyalgia rheumatica (PMR) were younger (73.4 +/- 6.3 versus 75.6 +/- 6.9 yr; p = 0.013) and had a longer delay to diagnosis (13.4 +/- 12.2 versus 8.3 +/- 10.0 wk; p = 0.013). One hundred thirty-one (54.6%) patients presented with severe ischemic manifestations. Abnormal temporal artery on physical examination (odds ratio, 2.25) and anemia at the time of disease diagnosis (odds ratio, 0.53) were found to be the best predictors for severe ischemic manifestations of GCA. Eighteen (7.5%) patients presented without overt ischemic manifestations of GCA. Patients younger than 70 years of age at the time of diagnosis had a longer delay to diagnosis and exhibited PMR more commonly than older patients. Our observations confirm the presence of different disease patterns of clinical presentation in GCA and emphasize the importance of an abnormal temporal artery on physical examination and anemia as factors that may predict the risk of severe ischemic complications related to GCA.

Influence of Traditional Risk Factors of Atherosclerosis in the Development of Severe Ischemic Complications in Giant Cell Arteritis

Because the prognosis of giant cell arteritis (GCA) is related to the development of ischemic complications, we sought to assess the possible influence of traditional risk factors of atherosclerosis in the development of severe ischemic complications of GCA. We conducted a retrospective study of patients with biopsy-proven GCA diagnosed from 1981 to 2001 at the single hospital for a well-defined population of almost 250,000 people. Patients were considered to have severe ischemic manifestations if they suffered visual manifestations, cerebrovascular accidents, jaw claudication, or signs of occlusive changes in large arteries of the extremities. Patients were assessed for the presence of hypercholesterolemia, hypertension, diabetes mellitus, and heavy smoking at the time of GCA diagnosis. The presence of traditional risk factors of atherosclerosis at the time of GCA diagnosis in this series of 210 patients increased significantly the risk of developing at least 1 of the severe ischemic complications (odds ratio [OR], 1.79; 95% confidence intervals [CI], 1.03-3.11; p = 0.04). Patients with traditional atherosclerosis risk factors had fever less commonly than the rest of GCA patients (5.2% vs. 16.0%; p = 0.01). GCA patients with hypertension exhibited a significantly increased risk of developing severe ischemic complications (OR, 1.80; 95% CI, 1.00-3.25; p = 0.05). The current study suggests that the presence of atherosclerosis risk factors at the time of diagnosis of GCA may influence the development of severe ischemic manifestations of the disease.

Fever in biopsy-proven giant cell arteritis: clinical implications in a defined population.

To assess the frequency and clinical features of biopsy-proven giant cell arteritis (GCA) patients who had fever at the time of diagnosis of the disease, and the relationship between fever, ischemic complications, and the systemic inflammatory response in GCA. A retrospective study of biopsy-proven GCA patients diagnosed between 1981 and 2001 was performed at the single referral hospital for a well-defined population in the Lugo region of northwest Spain. Patients were considered as having fever if the axillary temperature at the time of admission or during the followup prior to the onset of corticosteroid therapy was > or =38 degrees C. During the period of study, 21 (10%) of the 210 biopsy-proven GCA patients had fever. Two of them fulfilled criteria for fever of unknown origin. Patients with fever had a lower frequency of severe ischemic manifestations than the rest of biopsy-proven GCA patients. They also exhibited a more severe inflammatory disease, with significant abnormality in most laboratory variables, including higher elevation of erythrocyte sedimentation rate, lower values of hemoglobin, and higher proportion of patients with increased alkaline phosphatase. By logistic regression analysis, we observed that patients with fever had an increased risk of developing anemia (odds ratio [OR] 12.24). In contrast, a negative association between severe ischemic manifestations and fever was found (OR 0.41). Biopsy-proven GCA patients with fever constitute a subgroup of patients with more severe inflammatory response and less ischemic disease.

Incidence and Characteristics of Patients with Hand Ischemia after a Hemodialysis Access Procedure

The purpose of this study was to determine the clinical characteristics of chronic renal failure patients who developed hand ischemia in the limb carrying the dialysis angioaccess. A retrospective review of the charts of 352 patients who underwent 409 upper extremity arteriovenous access, and who were subsequently diagnosed as steal syndrome, was performed at the Emory University Hospital between February 1992 and January 1997. Hand ischemia occurred after 13 of 299 arteriovenous grafts (4.3%) and after 2 of 110 direct forearm arteriovenous fistulas (1.8%). Six patients developed ischemic manifestations immediately postoperatively, 2 in the first week, 4 after 1 month, and 1 after 1 year. Thirteen occurred in association with the primary access procedure. Two cases occurred following graft thrombectomy and outflow dilatation. Seven patients were mildly symptomatic with dialysis-induced pain, coldness, or numbness; 8 patients developed severe ischemic manifestations in the form of sensory loss in 3, severe intolerable pain with impalpable pulse in 3, and digital gangrene and amputation in 2, one of whom developed an unhealed amputation stump and required a higher amputation level with satisfactory healing of the revised stump. Three patients were treated conservatively, 6 by banding, 4 by ligation, 1 by embolization, and 1 by distal ligation and bypass operation. Clinical characteristics of patients with hand ischemia included long-standing insulin-dependent diabetes (10), chronic hypertension (12), peripheral arterial disease (14; 93.3%), coronary artery disease (8), and systemic lupus erythematosis (1). Severe peripheral arterial diseases are commonly found and may be markers for risk of hand ischemia after access surgery.